Diffuse Intrinsic Pontine Glioma

Rare Disease Highlight: Diffuse Intrinsic Pontine Glioma

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Rare Disease Highlight

By Anna Culbertson

Diffuse Intrinsic Pontine Glioma (DIPG) is an aggressive tumor developing from irregular growth of specific brain cells called glioma cells. The area of the brain primary affected is called the brainstem (specifically the pons), which is buried deep into the brain, making treatment very difficult 1. DIPG primarily affects children and makes up around 10-20% of all childhood brain tumors. The median age of those diagnosed with DIPG is 6-9 years old, although DIPG may affect older individuals2. Symptoms may include weakened strength, clumsiness, difficulty walking, and loss of balance 3. DIPG is usually fatal with a 5-year survival rate of 2%. There are no known causes of DIPG, but different individuals may have various chances of survival based upon age when diagnosed or the length of time between symptom presentation and diagnosis4,5.  The only effective treatment for this disorder is radiotherapy, although other treatments may be used experimentally, or to treat specific symptoms of DIPG 6. There is clearly a need for new drugs to be developed that target this rare disorder and help those who have it.  

References

  1. Buczkowicz P, Hawkins C. Pathology, Molecular Genetics, and Epigenetics of Diffuse Intrinsic Pontine Glioma. Frontiers in Oncology. 2015;5(147).
  2. Mathew RK, Rutka JT. Diffuse Intrinsic Pontine Glioma : Clinical Features, Molecular Genetics, and Novel Targeted Therapeutics. J Korean Neurosurg Soc. 2018;61(3):343-351.
  3. Hennika T, Becher OJ. Diffuse Intrinsic Pontine Glioma: Time for Cautious Optimism. J Child Neurol. 2016;31(12):1377-1385.
  4. dipg.org. Causes of DIPG 2021.
  5. Hoffman LM, Veldhuijzen van Zanten SEM, Colditz N, et al. Clinical, Radiologic, Pathologic, and Molecular Characteristics of Long-Term Survivors of Diffuse Intrinsic Pontine Glioma (DIPG): A Collaborative Report From the International and European Society for Pediatric Oncology DIPG Registries. Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 2018;36(19):1963-1972.
  6. Gallitto M, Lazarev S, Wasserman I, et al. Role of Radiation Therapy in the Management of Diffuse Intrinsic Pontine Glioma: A Systematic Review. Adv Radiat Oncol. 2019;4(3):520-531.

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